Recent deterioration

[Devonviolet]

I have an autoimmune liver disease called Nodular Regenerative Hyperplasia. This is caused by a primary immune condition called Common Variable Immune Deficiency. I have deteriorated slowly over 10 years and the phrase “ advanced liver disease” was used for the first time in September.

A few weeks later swelling occurred in my legs and abdomen. I guessed correctly this was ascites. I am on Propanol for Portal Vein Hypertension and now Spirolactone which has got rid of the fluid. I am awaiting a prescription of Renapro and trying to maintain a high protein and low salt diet.

Having looked on Google and this forum it feels like getting to this point, equates to falling off a cliff medically. I have seen articles saying 50% of patients are dead after a year and another saying 50% at two years. I am seeing a consultant just before Xmas. Will he be able to give me information on this? I deliberately hadn’t looked ahead and thought I would find out information as I needed it. There seems to be so little treatment available to counteract this situation and I know transplants rarely work with Primary Immune Patients. I’m hoping people here will let me know their experiences and any questions I should ask when I see the doctor the week before Xmas. Thanks

[Fibro2021]

As I remember earlier you wrote that you don't have any symptoms. Have your lab tests changed in any way lately? Perhaps low albumin could cause ascites?

[Devonviolet in reply to Fibro2021]

my albumin levels are a little low but they have been stable at that level for several years and my immunology team don’t seem concerned about them.

[Fibro2021 in reply to Devonviolet]

Portal hemodynamics may have worsened, and a little low level of albumin has become a background for the development of ascites. I also want to note sometimes we need to constantly balance between between a high protein diet on one side and the onset or worsening of symptoms of hepatic encephalopathy along with an increase in ammonia levels on the other side.

[Readlots]

It’s so hard to know how much information to ask for when you’re dealing with this type of illness. Lots of people prefer not to know their prognosis, just to deal with things as they arise. For me, I think the question would be what treatments / support is available to give me the best quality of life going forward.

[Alibaba80]

Do you have symptoms? Was your NRH diagnosed 10 years ago?

[Devonviolet in reply to Alibaba80]

I have had it for ten years. It would nice to know if other people had been stable with diuretics for a reasonable length of time. I have only been told recently that it is at an advanced stage, which came out of nowhere! Before that I was told there was only s slight change at the six monthly ultrasounds. I have Stage 1 varicies that are checked every two years by endoscopy. The only obvious symptom was the recent ascites which did clear with diuretics. No other obvious symptoms.

[Alibaba80 in reply to Devonviolet]

Thank you for your response. So you are able to eat as normal? And no weight loss?

They diagnosed it via biopsy when your LFTs were deranged?

In general you have been OK apart from the recent ascites? Do you maintain a healthy diet?

Do you take medication for the autoimmune disorder?

[Devonviolet]

Hi, I have recently lost some weight, but only a few pounds. In the past I have used Time Restricted Eating to maintain my weight. I have weekly sub cutaneous infusions of immunoglobulin for my primary immune disease, there is a part of my immune system that is missing and it took 30 years of infections before that was finally diagnosed. NRH is autoimmune and I’m on Propanalol and Spirolactone for that. My diet is anything but healthy at the moment! My comfort foods are cake and biscuits. But I am now on a low salt diet. So out go things like olives, bacon and smoked salmon as well. I was made aware of the need to maintain muscle when I saw a dietitian in October. As I am still shielding and have a lot of time at home since Covid, I have tried to do Joe Wicks type workouts five times a week. But I have changed some of the exercises to include ones that support muscle strength.

[Fibro2021 in reply to Devonviolet]

I just wanted to clarify that you are not entirely correct when you write that NRH is an autoimmune disease, although in your case it may be so. There are many reasons for the development of NRH. It's now considered more of a vascular disease of the liver. Propranolol is prescribed to reduce the risk of bleeding from esophageal varices in portal hypertension, spironolactone is a diuretic and prescribed for edema and ascites to reduce them . In your previous post, you wrote that you drink 2 units of alcohol per week. Although at this dose it's hardly worth considering alcohol as the main cause of liver disease, however, in liver diseases, all possible factors of liver damage, including alcohol, should be excluded. This can be compared to several arrows flying at the same target.

[Devonviolet]

You are so knowledgeable! Do you have a medical background? Or just had to up skill yourself because general practitioners have never heard of it? My immunology consultant told me it was an autoimmune disease. And since my diagnosis no one has ever told me not to have any alcohol. I assumed that I was destroying my own liver so an occasional drink wouldn’t matter. So I asked in May about alcohol and they said better not to, so I haven’t had any since. You would think that maybe a liver team at a big teaching hospital would give you some information at diagnosis, but they didn’t.

[Fibro2021 in reply to Devonviolet]

Yes, I had to educate myself. You hit the spot most of the doctors I deal with about this have never heard of NRH. They seem to happily diagnose cirrhosis only.

[mrob0823]

Hi Devon - I also have NRH and CVID. It's at an early stage with small varices, but as you mentioned, the NRH is auto-immune in our case versus others that get NRH for another reason. Did you get a fibroscan or biopsy done? My fibroscan was 7.4 showing minimal fibrosis/stiffness and my transjugular biopsy showed a portal venous system pressure of 5 hvpg. The biopsy also showed hepatoportal sclerosis. Was that mentioned in yours?

[Devonviolet in reply to mrob0823]

hello there, that is interesting, mine is more advanced.I have had it for 11 yrs I too have small varices , on Propanalol ( beta blocker) and Spironolactone( diuretic) for ascites. I had a biopsy 11 yrs ago and the last fibroscan was a long time ago. I have ultra sounds every 3 months and endoscopy every 2 years. I am seen at a big liver unit in the U.K. but obviously concerned about the recent ascites problem. As treatments seem limited when you reach this stage. Transplants don’t usually work well in CVID patients. I’ve never had my portal venous levels checked but ultrasounds seem to show P V hypertension. Maybe you are in another country or at the unit that has a different way of testing. I can’t do anything about it now but I feel let down by a system that said” it’s only a slight change “ each visit to you have

“ serious liver disease “ When I challenged this, the consultant said that most people would rather not know! Keep in touch! Nice to know there is someone out there with the same disease. Devon

[mrob0823 in reply to Devonviolet]

I'm being seen at Mass General Hospital in Boston. A Transjugular biopsy can measure your portal hypertension, which I had done a year ago. I'm surprised you get ultrasounds every 3 months. Does it show the NRH expanding? Also - does your hepatologist work with your immunologist? I'll ping you privately as this thread isn't specific to hepatic manifestations of CVID